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Aggressive_Recurrence_of_Primary_Hepatic_Epithelioid_Haemangioendothelioma_(Hehe)_after_Liver_Transplantation (2).pdf | 3.93 MB |
Introduction: HEHE is a rare neoplasm of vascular origin that that occurs in the liver, UNOS
reported a favorable outcome after liver transplantation in 110 patients with 1
and 5 years survival of 80% and 64%
Case Report: A 40 year old lady with history of hypertension, presented with three month
history of right upper abdominal pain with nausea, vomiting & significant
loss of weight associated with scleral icterus and progressive abdominal
distension.
Examination revealed jaundice, hepatomegaly and ascites
Serum bilirubin was 26.5 mg/dl , ALP was 552
Contrast Computed Tomography (CT) Abdomen and pelvic showed diffuse
infiltrative neoplastic process of the liver with a mass effect and stretching
of the hepatic and portal veins , in addition to bile duct dilatation.
Viral hepatitis markers were negative and serum Alpha fetoprotein was within
reference range.
Liver biopsy was consistent with HEHE, with positive endothelial markers (CD31,
CD34 & factor VIII-related antigen)
She underwent living Related Liver transplantation on June 2013, and was
discharged after 20 days with normal liver enzymes.
The explanted liver weighed 3222 grams. Gross and microscopic pathology showed
(fig1-7).
Four month later, she presented with severe abdominal pain and ascites with
elevated liver enzymes, CT showed numerous variable size non enhancing tumors
associated with small amount of ascites. Hepatic veins, artery and portal vein
are patent.
Liver biopsy confirmed disease recurrence, she received supportive treatment
and unfortunately she died 2 weeks later.
Conclusion: HEHE can have rapid and aggressive recurrence after liver transplantation.